skip to Main Content

As situation around COVID-19 continues to develop we, together with other PAGs, are asking clinicians questions and here are some answers we think may be useful. These come from three different clinicians (children and adult services) and we hope that they will add some clarity. If you have further questions please contact us on [email protected] and we will pass these on the clinical panel.

There are still many people who have not received NHS letters emails or texts stating they are in the ‘extremely vulnerable’ group and can’t access extra support.

We know of occasions where GPs are telling people that they can’t send the letters and that the neuromuscular team should be doing this. Unsurprisingly some neuromuscular services do not have the capacity to respond to these requests.

Inevitably, lots of people and their families are very concerned about this as they are shielding and are then unable to access the services that are available and be given flexible working or time off work.

Q1. Could you share what you are doing at your centres and also what you think is being done elsewhere in the neuromuscular clinical community?
A 1.1 Here in Newcastle we are sending out an advice letter to all our patients.
A 1.2 GOSH WILL START 8th of April.
A 1.3 We have created a list of 2500 extremely vulnerable patients attending our service, no names but NHS numbers, which our Trust has forwarded to NHS England, I have done the same for our Scottish patients and sent CHI number to NHS Scotland. These patients should get a text, we have also written to these patients directly advising them to shield. If they have not yet received our letter by email, they should check their junk folder.
(NOTE FROM TREATSMA – We have discovered that whilst your neuromuscular team may write a letter, you still should contact GP and make sure that GP adds your number to the extremely vulnerable NHS list. GPs may not be aware that they must do this because they may not have been told. Please call them and explain that they should do it ASAP. Neuromuscular teams do not actually have a way to do this. You may get a letter from neuromuscular team, but it does not mean you are registered on the vulnerable list. Check with your GP!)

Q2. What is the protocol for using NIV if dealing with the coronavirus? Should you increase pressure or rate or other settings?
A 2.1 This need to be addressed by the respiratory team.
A 2.2 You need to ask your respiratory expert for more detailed advice. However, you should not alter your NIV settings but it is safe to use your NIV more frequently for example during the day time rather than night time alone

Q3. Are people on NIV more likely to get a severe case than a mild one?
A 3.1 There is currently no data to support this but it is likely because of already impaired respiratory function.
A 3.2 We do not know why some people get this virus more severely than others. We have no data for people on NIV, probably NIV per se does not increase the risk of the severe cytokine reaction but someone on NIV may struggle with their breathing more if they develop a cough or pneumonia.

Q4. If admitted to hospital, would people on NIV be given oxygen treatment, or would that only be provided if they are admitted to critical care?
A 4.1 Oxygen can be provided even outside critical care.
A 4.2 People do not need to be in ITU to receive oxygen treatment.

Q5. As we know admittance to critical care will depend on prognosis, what are the influencing factors on prognosis?
A 5.1 Influencing factors on prognosis will be life expectancy, impaired cardiac and/or respiratory function, nutritional status, degree of disability in addition to covid-19 specific morbidity and level of organ involvement
A 5.2 The virus causes a pneumonia and intense inflammatory reaction. It is the extent of this secondary inflammatory reaction that causes severe disease. Inflammatory markers such as CPR, ESR, IL6, Ferritin and markers for thrombosis are raised. The blood count is also abnormal with low white cells. Prognosis depends on the level of these markers in the blood, the degree of lung involvement on XRAY. Another poor prognostic feature is the presence of acute inflammation in the heart. The inflammatory reaction leads to multi-organ failure which is a poor prognostic sign

Q6. Is there any evidence that people on NIV will be less likely to benefit quickly from critical care treatment?
A 6.1 It is likely that people with respiratory muscle weakness and on NIV may require longer period of time to recover from critical care treatment and also deteriorate in their global function following a prolonged admission.
A 6.2 I WOULD SAY THAT AT THE MOMENT WE ARE GATHERING EVIDENCE ON HOW PATIENTS WITH NEUROMUSCULAR DISEASES ALREADY ON VENTILATED ARE RESPONDING TO COVID-19, SO AT THE MKMENT THERE IS NO EVIDENCE, BUT CLEARLY THE PROGNOSIS WILL BE MORE GUARDED IN PATIENTS WITH SEVERE RESPIRATOIRY INVOLVEMENT FOR COVID 19 AND ANY OTHER RESPIRATORY INFECTION
A 6.3 It is important to be aware that when people with Covid-19 are admitted to ITU their condition is extremely critical. Only 50% of people will survive ITU and those that do will have longstanding respiratory problems. People who are intubated are likely to be paralysed and ventilated for 2-3 weeks.

Q7. “If I have symptoms, and am finding it harder to breath, at what point should I go to hospital? Should I try altering my use of NIV first?”
A 7.1 If you develop symptoms you should call 111.
A 7.2 Call 111 for advice

Q8. I know that some people with mild CMT have been told they don’t need to shield. If I have cmt1a and get sleep apnoea, does this put me in the highest risk group in your consensus clinical advice and should I be shielding and encouraging my household to shield as well?
A 8.1 There is no increased risk from OSA but it will also depend on other risk factors and co-morbidities. Your doctor is best placed to assess the individual risk. additional information is available here https://www.brit-thoracic.org.uk/media/455098/osa-alliance-cpap-covid-19-advice-20-3-20-v10.pdf
A 8.2 THE MAIN DETEINANT FACTOR IS THE RESPIRATORY FUNCTION. IF RESPIRATORY FUNCTION IS NORMAL, THE USUAL PRECAUTIONS ARE RECOMMENDED; IF NOT NORMAL OR YOU ARE NOT SURE, SELF ISOLATION WILL BE SAFER. DISCUSS WITH YOUR DR.
A 8.3 The British Thoracic Society have issued a statement that obstructive sleep apnoea requiring CPAP without any other co-morbidities such as obesity, heart disease or diabetes, is not a risk factor.

Q9. With regards to patients receiving Spinraza, are treatment centres wanting patients due for treatment to be tested for covid 19 before attending for treatment? If so, how is this arranged?
A 9.1 We are not asking patients to be tested, but if unwell (even if not covid-19 positive) and even more if Covid-19 positive we would generally advice to post-pone the administration of Spinraza.

Q10. Several our members [DMD Pathfinders] are already taking herbal supplements such as Echinacea and Elderberry extract to boost the immune system (based on papers published e.g. https://www.ncbi.nlm.nih.gov/pubmed/15080016). We know that a cytokine storm can be fatal to those with Covid-19, so would you recommend patients avoid these immune boosting remedies? Or to stop them if they get symptoms?
A10.1 The decision and the management to take unlicensed medications resides with the people who take them, and it would be inappropriate for us to comment

Q11. Those that have the neuromuscular care plans, is there a way of adding extra on to them to cover for hospital admissions with covid19?
A11.1 The neuromuscular care plan should be updated regularly and should provide key information for professionals to be able to take decisions in case of unplanned admission.
A11.2 I don’t think that there is anything specific to be added apart from ensuring that the neuromuscular clinicians is contacted in case of admission. This should however already be stated. Similarly guidance regarding changing the ventilator circuit and filters if tested COVID-19 positive should happen anyway when admitted to hospital.
A11.3 As indicated, there is not necessarily anything specific or – from an infection control perspective- different of this corona virus from other virally induced conditions, so there should not be a different path compared to the path regarding hospital admission for a virally contagious disease

Q12. There are some young men living independently in care homes whose parents cannot get in to see them or to check on their wellbeing, whose staff would not know whether to up hours on bipap etc if the person was feeling unwell, is there any information around that parents can give to the care homes, as a plan to follow in case of them feeling unwell? And going on from this, if a DMD young man had covid19, and was in a care home and hospital don’t or can’t admit said person, for whatever reasons is there a plan of action that staff could follow if the patient was to stay in the care home scenario especially without parent involvement? Bearing in mind these staff aren’t nurses generally. ? care home staff should contact the home ventilation team to ask for specific advice on the ventilator. Contact details should be on each ventilator.
A12.1 I would follow the advice of similar situations in which patients were not very well. It is unlikely this would be the first time in the life of these patients in the care home to be unwell, so I would implement the path previously followed in similar situations, and if in doubt, the respiratory queries need to be raised specifically with the individual respiratory team. In any event, if patients are unwell and concerned, they should contact NHSE

Q13. If families haven’t completed the neuromuscular care plans up to now but want to do them and are using the template available on the MDUK website, which needs input from the specialist centres, should this be sent to the clinician or can the family care advisor help with this?
A13.1 It depends on the professional background of the family care advisor. A care plan usually requires input from a multidisciplinary team. The care advisor or nurse specialist can help draft the care plan and coordinate input from the various professionals and patient.
A13.2 An FCA can help to coordinate the care plan, or a nurse specialist; or a neuromuscular or respiratory physician or therapist. Different centres will have a different structure

Back To Top